The DOI matches its Crossref record; 24 of the 24 references it cites were found in a registry or an index.
Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates.
Open accessAbstract
Although the gene defect responsible for Huntington disease (HD) has recently been identified, the pathogenesis of the disease remains obscure. One potential mechanism is that the gene defect may lead to an impairment of energy metabolism followed by slow excitotoxic neuronal injury. In the present study we examined whether chronic administration of 3-nitropropionic acid (3-NP), an irreversible inhibitor of succinate dehydrogenase, can replicate the neuropathologic and clinical features of HD in nonhuman primates. After 3-6 weeks of 3-NP administration, apomorphine treatment induced a significant increase in motor activity as compared with saline-treated controls. Animals showed both choreiform movements, as well as foot and limb dystonia, which are characteristic of HD. More prolonged 3-NP treatment in two additional primates resulted in spontaneous dystonia and dyskinesia accompanied by lesions in the caudate and putamen seen by magnetic resonance imaging. Histologic evaluation showed that there was a depletion of calbindin neurons, astrogliosis, sparing of NADPH-diaphorase neurons, and growth-related proliferative changes in dendrites of spiny neurons similar to changes in HD. The striosomal organization of the striatum and the nucleus accumbens were spared. These findings show that chronic administration of 3-NP to nonhuman primates can replicate many of the characteristic motor and histologic features of HD, further strengthening the possibility that a subtle impairment of energy metabolism may play a role in its pathogenesis.
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Cites
Neuropathological Classification of Huntingtonʼs Disease
VONSATTEL, J.-P., MYERS, R. H., STEVENS, T. J., FERRANTE, R. J., BIRD, E. D., & RICHARDSON, E. P. (1985) · Journal of Neuropathology and Experimental Neurology · doi:10.1097/00005072-198511000-00003 · Cited by 2,227
Self-citationReplication of the neurochemical characteristics of Huntington's disease by quinolinic acid
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Self-citationNovelli, A., Reilly, J. A., Lysko, P. G., & Henneberry, R. C. (1988) · Brain Research · doi:10.1016/0006-8993(88)90765-2 · Cited by 971
Beal, M. F. (1992) · Annals of Neurology · doi:10.1002/ana.410310202 · Cited by 961
Self-citationAlternative excitotoxic hypotheses
Albin, R. L., & Greenamyre, J. T. (1992) · Neurology · doi:10.1212/wnl.42.4.733 · Cited by 574
Chronic quinolinic acid lesions in rats closely resemble Huntington's disease
Beal, M., Ferrante, R., Swartz, K., & Kowall, N. (1991) · The Journal of Neuroscience · doi:10.1523/jneurosci.11-06-01649.1991 · Cited by 499
Self-citation3-Nitropropionic Acid - Exogenous Animal Neurotoxin and Possible Human Striatal Toxin
Ludolph, A. C., He, F., Spencer, P. S., Hammerstad, J., & Sabri, M. (1991) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/s0317167100032212 · Cited by 485
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Self-citationNMDA Receptor Losses in Putamen from Patients with Huntington's Disease
Young, A. B., Greenamyre, J. T., Hollingsworth, Z., Albin, R., D'Amato, C., Shoulson, I., & Penney, J. B. (1988) · Science · doi:10.1126/science.2841762 · Cited by 395
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Cited by
Mitochondrial Dysfunction and Oxidative Stress in Neurodegenerative Diseases
Lin, M. T., & Beal, M. F. (2006) · Nature · doi:10.1038/nature05292 · Cited by 6,455
Self-citationMitochondrial Dysfunction in Neurodegenerative Diseases
Johri, A., & Beal, M. F. (2012) · The Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.112.192138 · Cited by 806
Self-citationThe Role of Excitotoxicity in Neurodegenerative Disease
Doble, A. (1999) · Pharmacology & Therapeutics · doi:10.1016/s0163-7258(98)00042-4 · Cited by 723
Weydt, P., Pineda, V. V., Torrence, A. E., Libby, R. T., Satterfield, T. F., Lazarowski, E. R., Gilbert, M. L., Morton, G. J., Bammler, T. K., Strand, A. D., et al. (2006) · Cell Metabolism · doi:10.1016/j.cmet.2006.10.004 · Cited by 562
Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease
Ferrante, R. J., Andreassen, O. A., Jenkins, B. G., Dedeoglu, A., Kuemmerle, S., Kubilus, J. K., Kaddurah-Daouk, R., Hersch, S. M., & Beal, M. F. (2000) · The Journal of Neuroscience · doi:10.1523/jneurosci.20-12-04389.2000 · Cited by 522
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Metadata
- Authors
- Brouillet, E., Hantraye, P., Ferrante, R. J., Dolan, R., Leroy-Willig, A., Kowall, N. W., & Beal, M. F.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 1995-07-18
- Volume
- 92
- Issue
- 15
- Pages
- 7105-7109
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W1978190113
- Cited by
- 485
- References
- 24
- FWCI
- 5.84
- Type
- journal article
- Language
- en
- Open access
- green
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- Brouillet, E., Hantraye, P., Ferrante, R. J., Dolan, R., Leroy-Willig, A., Kowall, N. W., & Beal, M. F. (1995). Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.92.15.7105
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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